Rare case of acute renal failure due to Hemolytic-uremic Syndrome (HUS) in third trimester pregnancy

Introduction HUS is a rare life-threatening thrombotic mikroangiopathy characterized by acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia. P-aHUS occurs in 1 out of 25000 pregnancies, mostly in the postpartum period. Most patients suffer from neurologic injury, renal impairment, and multiorgan failure. 60 to 70% of aHUS develope end-stage renal disease. Therapy involves plasma exchange and the use of anti-C5 therapy (Eculizumab) to induce terminal complement blockade .

Case A 35-year-old G1P0 at 36+6 weeks of gestation presented in our delivery room with headache and upper abdominal pain. As we determined hypertensive blood pressure and hyperreflexia we indicated magnesium- and Ebrantil-perfusor. Due to laboratory values performed urgent casearean section in suspicion of HELLP-syndrome. Afterwards the patient was transferred to our intensive care unit. She developed acute renal failure with anuria and hyperkalemia, platelets counts dropped, transaminases and LDH increased, vigilance was reduced. P-aHUS was considered, plasmapheresis was performed immediately. Next day we started treatment with Eculizumab 900mg, administered on day 1,5 and 10. The patient obtained antibiotic treatment because of therapy with monoclonal antibody. Additionally Bexsero was given, Menveo was already obtained. We achieved nearly complete recovery of renal parameters within 14 days. Patient was discharged in good condition two weeks postpartum. We recommended Eculizumab lifelong 1200mg/14 days,

Conclusion Since typical symptomes of p-aHUS resemble to HELLP syndrome, TTP, and acute fatty liver of pregnancy early diagnosis is challenging. To improve outcomes early diagnosis and early management by an interdisciplinary team with prompt plasmapheresis und Eculizumab is crucial.

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Publication History

Article published online:
07 October 2020